Background: The AHOPCA (Asociación de Hemato-Oncología Pediátrica de Centro América) Wilms tumor (WT) treatment guidelines, adapted from NWTS-4 and -5 and recommending upfront nephrectomy (UN), were modified to include 4 to 6 weeks of preoperative chemotherapy (POC) in frail patients with advanced disease. We aim to describe the outcomes of this implemented approach. Procedure: A retrospective analysis of prospectively collected data from newly diagnosed patients with unilateral WT between 2012 and 2018. Results: Of 353 eligible patients, 247 (70%) received POC. POC group had higher initial tumor volume (median 590 ml vs. 308 ml, p<0.0001) and more stage IV disease (30% vs. 3%, p<0.0001) compared to UN group. During POC, there were 4 deaths, 14 abandonments and 11 progressive diseases. After response assessment, 62% showed partial tumor response with median tumor volume reduction to 257 ml. Two-hundred eighteen (88.2%) underwent nephrectomy. Tumor rupture/spillage occurred in 17.9% of UN and 18.8% of POC group; Local stage III was found in 55% of UN and 68.3% of POC group. Anaplasia (focal and diffuse) was present in 13.6% (17% UN and 11.9% POC). The 5-year abandonment-sensitive event-free survival (asEFS) and abandonment-sensitive overall survival (asOS) rates were 69%±2.5% and 75%±2.4%, respectively. The asEFS rates were 75%±4.3% for UN and 66%±3.0% for POC (p=0.07). Conclusions: Many AHOPCA patients presented with unresectable advanced disease, necessitating the use of POC. POC effectively reduced tumor volume and facilitated safe surgery in fragile patients. Adopting the standard SIOP WT neoadjuvant approach could enhance staging and risk stratification in AHOPCA.

Background and Aims. Since the war in Ukraine which began on February 2022, many pediatric oncology centers have welcomed patients from Ukraine. To understand the needs of patients and families arriving at our hospital, an anonymous questionnaire investigated the families’ backgrounds, feelings and impressions about hospitality and care. Methods Twenty items investigated how patients had reached Italy, from whom they received help (logistically/economically); emotions regarding their statusas war refugees; knowledge, expectations, and opinions about Italy and Italians; the quality of medical care received and relationships with the healthcare staff; suggestions to improve assistance. Results Questionnaires were completed by 19/32 patient/parents in the time interval May-November 2022 in two different pediatric-oncology centers in the north of Italy. Most families had reached Italy (58%) and received medical care (95%) thanks to the help of Charities and the Italian Public Health Care System. Many of them (69%) declared themselves to be satisfied by the assistance. The Italian population appeared friendly (95%) and generous (58%). The improvement of their stay correlated with the positive outcome of their children (15%), the presence of the whole family (15%), the end of the war (10%), and the overcoming of language barriers (10%) Conclusions. Taking care of children from a different country suffering the traumatic experience of fleeing their country in addition to the equally traumatic condition of cancer disease, is a huge task. Our questionnaire aimed at obtaining a better understanding of families’ conditions, not at bridging the relational gap due to different culture and experiences.

Introduction. The H3K27M-mutant diffuse midline glioma (DMG) was first included in the WHO Classification of CNS (central nervous system) tumors in 2016, and confirmed in its fifth edition. The biological behavior and dismal prognosis of this tumor resemble DIPG (diffuse intrinsic pontine gliomas). Homogeneously-treated series are rarely reported. Methods. From 2016 onwards, we treated patients with DMG with radiotherapy and concomitant/adjuvant nimotuzumab/vinorelbine, plus re-irradiation at relapse, as already done for DIPG (DOI10.1007/s11060-014-1428-z). Results. We treated nine patients, seven females, median age at diagnosis of 13 years-old. Tumor sites were: thalamic in five cases, pontocerebellar in two, pineal in one, and paratrigonal with nodular/leptomeningeal dissemination in one. Three patients were biopsied, and six had partial tumor resections. Central review of the pathologists’ diagnoses was performed. The median time to local progression was 12.7 months, and the median overall survival was 17.8 months. Six patients died of tumor progression, one of cerebral bleeding whose tumor was progressing. Two were alive, one in continuous remission, the other after a relapse, at 38.6 and 46.3 months after diagnosis, respectively. Progression-free survival was 33.3% at one year. Overall survival was 88.9%, 33.3% and 22.2% at 1, 2 and 3 years, respectively. Conclusions. This is one of only a handful of reports on homogeneously-treated series. The results obtained are comparable with those seen in patients with DIPG. Given the phenotypically- and molecularly-defined setting of DMG and severe outcome in this orphan population, they should be treated and included in registries and protocols of DIPG.

During SARS-CoV-2 pandemic, oncologists manage patients at higher risk of having a severe course of this infection. This raises new questions about their correct management, as well as the difficulty of distinguishing tumor/treatments complications from those related to Coronavirus disease 2019 (COVID-19). We report a case of an 11 year-old boy undergoing treatment for T cell lymphoblastic lymphoma who experienced a prolonged SARS-CoV-2 infection. Oncological therapy was continued without significant changes compared to the initially planned treatment. No relevant complications occurred. COVID-19 convalescent plasma (CP) was administered, resulting in a positive antibody titer after 24 days.

Introduction. Mutations of the APC (adenomatous polyposis coli) gene correlate mainly with familial adenomatous polyposis (FAP), but can occasionally be pathogenic for medulloblastoma (MBL) WNT subtype as well, the course of which has only recently been described. Methods. We retrospectively retrieved all patients with documented germline APC mutations and a centrally-reviewed diagnosis of MBL to examine the outcome of their MBL, late effects of its treatment, and further oncological events. Results. Between 2007-2016 we diagnosed and treated 6 patients, all with a pathogenic APC variant mutation, who all had MBL, classic histotype. None had metastatic disease. All patients were in complete remission a median 65 months after treatment with craniospinal irradiation at 23.4 Gy, plus a boost on the posterior fossa/tumor bed up to 54 Gy, followed by cisplatin/carboplatin, lomustine and vincristine for a maximum of 8 courses. Five of 6 diagnostic revised MRI were suggestive of the WNT molecular subgroup typical aspects. Four of 6 patients had a positive family history of FAP, while gastrointestinal symptoms prompted its identification in the other 2 cases. Four patients had developed other tumors (desmoid, MELTUMP, melanoma, pancreatoblastoma, thyroid Tir3) from 5 to 7 years after MBL. Discussion. Our data confirm a good prognosis for patients with MBL associated with FAP. Patients’ secondary tumors may or may not be related to their syndrome or treatment, but warrant adequate attention when planning shared guidelines for these patients.

Background: Children and adolescents with cancer are at risk of a poor health-related quality of life. Exercise interventions to enhance movement may be a valid strategy for managing some symptoms, including fatigue. Methods: Forty-four consecutive patients (20 females; aged 5-21 years old, median 15.5 years), without any contraindications significantly limiting their movements were invited to join an in-hospital 6-week supervised exercise program, and asked afterwards to complete the PedsQL-4.0 quality of life Generic Core Scales and the PedsQL Multidimensional Fatigue Scale. The program consisted of personalized workout sessions of aerobic, resistance and flexibility exercises. The results obtained on the scales were compared between patients who engaged in the exercise program (GYM group, n=21) and those who did not (No-GYM, n=23), with the aim of examining the different dimensions of health-related quality of life (physical, emotional, cognitive, social) and fatigue (general, sleep/rest, cognitive) comparing the two groups. Results: For 43 of 44 patients, being diagnosed with cancer initially prompted a drop-out from previous physical exercise or sports routines despite no contraindications to their continuation. After 6 weeks, the scores for patients in the GYM group showed a statistically significant better perceived emotional functioning, and a possible indication of improved social functioning compared with the No-GYM group. Conclusion: These findings suggest that to exercise improves the satisfaction of children and adolescents with cancer with their physical, mental and social functioning. It is worth further investigating the value of systematically including exercise workouts in their routine cancer practices.